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https://www.readbyqxmd.com/read/28798943/a-fatal-case-of-erdheim-chester-disease-with-hepatic-involvement
#1
Gokulakrishnan Balasubramanian, Alexandra Modiri, Marina Affi, Catherine E Hagen, Bjorn Batdorf, Kiyoko Oshima, Laura Michaelis, Kia Saeian
Erdheim-Chester disease (ECD) is a rare form of systemic histiocytosis, typically presenting with striking osseous involvement characterized by bilateral osteosclerosis and involvement of organs such as the lung, pituitary gland, heart, and brain. Liver involvement with ECD is extremely uncommon. We report a 56-year-old woman presenting with newly diagnosed cirrhosis and signs concerning for intra-abdominal malignancy, including omental caking and peritoneal thickening. Liver biopsy demonstrated xanthogranulomatous infiltration from ECD...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28796076/factors-affecting-mortality-and-resource-use-for-hospitalized-patients-with-cirrhosis-a-population-based-study
#2
Phunchai Charatcharoenwitthaya, Ngamphol Soonthornworasiri, Khemajira Karaketklang, Kittiyod Poovorawan, Wirichada Pan-Ngum, Watcharasak Chotiyaputta, Tawesak Tanwandee, Kamthorn Phaosawasdi
Hospitalizations for advanced liver disease are costly and associated with significant mortality. This population-based study aimed to evaluate factors associated with in-hospital mortality and resource use for the management of hospitalized patients with cirrhosis.Mortality records and resource utilization for 52,027 patients hospitalized with cirrhosis and/or complications of portal hypertension (ascites, hepatic encephalopathy, variceal bleeding, spontaneous bacterial peritonitis, or hepatorenal syndrome) were extracted from a nationally representative sample of Thai inpatients covered by Universal Coverage Scheme during 2009 to 2013...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28795995/toxic-myocarditis-caused-by-acetaminophen-in-a-multidrug-overdose
#3
Maxime Gosselin, Yann Dazé, Pascal Mireault, Marie Crahes
We report the case of an 18-year-old woman with personality disorders who was hospitalized a few hours after suicidal ingestion of acetaminophen, quetiapine, acetylsalicylic acid, and ethanol. Twelve hours after admission, severe liver damage was evident, but the patient was stable and awaiting hepatic transplantation. Electrolytes were successfully controlled. The condition of the liver stabilized. Cardiac biomarkers then deteriorated unexpectedly. Localized ST-segment elevations were noted on electrocardiogram, but angiography ruled out myocardial infarction...
August 9, 2017: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/28792999/clinical-features-of-refractory-ascites-in-outpatients
#4
Wanda Regina Caly, Rodrigo Martins Abreu, Bernardo Bitelman, Flair José Carrilho, Suzane Kioko Ono
OBJECTIVES: To present the clinical features and outcomes of outpatients who suffer from refractory ascites. METHODS: This prospective observational study consecutively enrolled patients with cirrhotic ascites who submitted to a clinical evaluation, a sodium restriction diet, biochemical blood tests, 24 hour urine tests and an ascitic fluid analysis. All patients received a multidisciplinary evaluation and diuretic treatment. Patients who did not respond to the diuretic treatment were controlled by therapeutic serial paracentesis, and a transjugular intrahepatic portosystemic shunt was indicated for patients who required therapeutic serial paracentesis up to twice a month...
July 2017: Clinics
https://www.readbyqxmd.com/read/28779790/management-of-renal-dysfunction-in-patients-with-liver-cirrhosis-role-of-pretransplantation-hemodialysis-and-outcomes-after-liver-transplantation
#5
Ashok Thorat, Long-Bin Jeng
Patients with end-stage liver disease (ESLD) who develop hepatorenal syndrome (HRS) have very high mortality rates. For patients with HRS type I, median survival without specific therapy is only 2 weeks. Due to worsening clinical condition in such patients secondary to uremia and hepatic disease, some form of renal replacement therapy (RRT), either intermittent hemodialysis IHD or continuous veno-venous hemodialysis (CVVHD), must be instituted. However, the literature regarding the survival benefits of the hemodialysis for the worsening renal failure in liver cirrhotic patients remains limited...
December 2016: Seminars in Vascular Surgery
https://www.readbyqxmd.com/read/28771246/diagnosis-and-treatment-of-tyrosinemia-type-i-a-us-and-canadian-consensus-group-review-and-recommendations
#6
REVIEW
Jeffrey M Chinsky, Rani Singh, Can Ficicioglu, Clara D M van Karnebeek, Markus Grompe, Grant Mitchell, Susan E Waisbren, Muge Gucsavas-Calikoglu, Melissa P Wasserstein, Katie Coakley, C Ronald Scott
Tyrosinemia type I (hepatorenal tyrosinemia, HT-1) is an autosomal recessive condition resulting in hepatic failure with comorbidities involving the renal and neurologic systems and long term risks for hepatocellular carcinoma. An effective medical treatment with 2-[2-nitro-4-trifluoromethylbenzoyl]-1,3-cyclohexanedione (NTBC) exists but requires early identification of affected children for optimal long-term results. Newborn screening (NBS) utilizing blood succinylacetone as the NBS marker is superior to observing tyrosine levels as a way of identifying neonates with HT-1...
August 3, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28769581/clinical-utility-of-nitisinone-for-the-treatment-of-hereditary-tyrosinemia-type-1-ht-1
#7
REVIEW
Anibh Martin Das
Medical therapy for hereditary hepatorenal tyrosinemia (hereditary tyrosinemia type 1, HT-1) with nitisinone was discovered incidentally, and is a by-product of agrochemistry. It blocks the catabolic pathway of tyrosine, thereby leading to a reduction in the accumulation of toxic metabolites in HT-1. It has to be combined with a low-protein diet supplemented with amino acid mixtures devoid of tyrosine and phenylalanine. This treatment option has completely changed the clinical course of patients suffering from HT-1 who used to die in the first few months to years of life from liver failure, renal dysfunction, and/or hepatocellular carcinoma (HCC)...
2017: Application of Clinical Genetics
https://www.readbyqxmd.com/read/28764217/performance-of-disease-specific-scoring-models-in-intensive-care-patients-with-severe-liver-diseases
#8
Maged T El-Ghannam, Moataz H Hassanien, Mohamed D El-Talkawy, Abdel Aziz A Saleem, Amal I Sabry, Hoda M Abu Taleb
INTRODUCTION: Egypt has the highest prevalence of Hepatitis C Virus (HCV) in the world, estimated nationally at 14.7%. HCV treatment consumes 20% ($80 million) of Egypt's annual health budget. Outcomes of cirrhotic patients admitted to the ICU may, in fact, largely depend on differences in the state of the disease, criteria and indications for admission, resource utilization, and intensity of treatment. AIM: The aim of the present study was to evaluate the efficacy of liver specific scoring models in predicting the outcome of critically ill cirrhotic patients in the ICU as it may help in prioritization of high risk patients and preservation of ICU resources...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28763855/-pathophysiological-basis-of-portal-hypertension-and-the-new-concept-of-acute-kidney-injury-in-patients-with-liver-cirrhosis
#9
J B Wang
Recently, the International Club of Ascites (ICA) has developed a new expert consensus on the diagnosis and treatment of acute kidney injury (AKI) in patients with liver cirrhosis, which reflects the new concept of AKI management in patients with liver cirrhosis. This consensus emphasizes the increase in the absolute value of serum creatinine (SCr) and establishes a new staging system for AKI, which may help to evaluate disease progression and recovery. In addition, the new management concept also emphasizes that when AKI progresses to stage 2/3 or still progresses after comprehensive treatment, a diagnosis can be made and vasoconstrictors and albumin can be used as long as the patient meets the other diagnostic criteria for hepatorenal syndrome, regardless of SCr level...
June 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28763843/-diagnostic-value-of-serum-cystatin-c-for-acute-kidney-injury-in-patients-with-liver-cirrhosis
#10
J J Cai, J Zhou, T Han, Y L Zhang, H P Liu, Y C Fu
Objective: To determine the diagnostic value of serum cystatin C (Cys C) for acute kidney injury (AKI) in patients with liver cirrhosis. Methods: Serum Cys C levels in 150 liver cirrhosis patients (88 AKI and 62 non-AKI patients) were measured by the Particle-Enhanced Nephelometric Immuno-Assay. The accuracy of serum Cys C for the diagnosis of AKI in liver cirrhosis was evaluated by the ROC curve. Results: Liver cirrhosis patients with AKI had significantly higher serum Cys C levels [2.37 (1.75-2.83) mg/L] than those without AKI [0...
May 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28763340/rifaximin-for-the-prevention-of-spontaneous-bacterial-peritonitis-and-hepatorenal-syndrome-in-cirrhosis-a-systematic-review-and-meta-analysis
#11
Faisal Kamal, Muhammad Ali Khan, Zubair Khan, George Cholankeril, Tariq A Hammad, Wade M Lee, Aijaz Ahmed, Bradford Waters, Colin W Howden, Satheesh Nair, Sanjaya K Satapathy
Prophylactic antibiotics have been recommended in patients with a previous history of spontaneous bacterial peritonitis (SBP). Recently, there has been interest in the use of rifaximin for the prevention of SBP and hepatorenal syndrome (HRS). We conducted a meta-analysis to evaluate this association of rifaximin. We searched several databases from inception through 24 January 2017, to identify comparative studies evaluating the effect of rifaximin on the occurrence of SBP and HRS. We performed predetermined subgroup analyses based on the type of control group, design of the study, and type of prophylaxis...
July 31, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28755198/remaining-challenges-in-the-treatment-of-tyrosinemia-from-the-clinician-s-viewpoint
#12
Grant A Mitchell, Hao Yang
This chapter provides a clinical perspective on the challenges that stand between current clinical practice and a cure for hepatorenal tyrosinemia (HT1). HT1 has been transformed in the last 50 years from an aggressive often undiagnosed childhood disease causing liver failure or liver cancer, with infant death in most patients, to a condition that is detectable at birth, and for which treatment with nitisinone (NTBC) and diet can prevent detectable liver or kidney abnormalities. What challenges remain? The properties of the affected metabolic pathway and the broad spectrum of severity seen in untreated patients are incompletely understood but potentially important for patients...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28755196/the-qu%C3%A3-bec-ntbc-study
#13
Fernando Alvarez, Suzanne Atkinson, Manon Bouchard, Catherine Brunel-Guitton, Daniela Buhas, Jean-François Bussières, Josée Dubois, Daphna Fenyves, Paul Goodyer, Martyne Gosselin, Ugur Halac, Patrick Labbé, Rachel Laframboise, Bruno Maranda, Serge Melançon, Aicha Merouani, Grant A Mitchell, John Mitchell, Guy Parizeault, Luc Pelletier, Véronique Phan, Jean-François Turcotte
In this chapter we describe the current Quebec NTBC Study protocol. Quebec's unique characteristics have influenced the development of the protocol, including a high prevalence of hepatorenal tyrosinemia (HT1), universal newborn screening for HT1, availability of treatment with nitisinone (NTBC) and special diet, a large territory, where HT1 treatment is coordinated by a small number of centers. Screened newborns are seen within 3 weeks of birth. Patients with liver dysfunction (prolonged prothrombin time and/or international normalized ratio (INR) provide sensitive, rapidly available indicators) are treated by NTBC and special diet...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28755193/hepatorenal-tyrosinemia-in-mexico-a-call-to-action
#14
Isabel Ibarra-González, Cecilia Ridaura-Sanz, Cynthia Fernández-Lainez, Sara Guillén-López, Leticia Belmont-Martínez, Marcela Vela-Amieva
Hepatorenal tyrosinemia is a treatable metabolic disease characterized by progressive liver failure, renal damage and pronounced coagulopathy. Its clinical diagnosis is difficult because of its low prevalence and heterogeneous symptoms. In developed countries, expanded newborn screening, based on succinylacetone quantification by tandem mass spectrometry, has been very valuable in the early detection of hepatorenal tyrosinemia, providing the opportunity for rapid treatment of affected patients. In developing countries without systematic expanded newborn screening, however, diagnosis and treatment of this disease remain major challenges, as genetic diseases in these countries are not a health priority and there are few referral centers for infants with inherited errors of metabolism...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28755190/diagnosing-hepatorenal-tyrosinaemia-in-europe-newborn-mass-screening-versus-selective-screening
#15
Anibh M Das, Sebene Mayorandan, Nils Janzen
Hepatorenal tyrosinaemia (HT1) is a serious condition that used to be fatal before the advent of nitisinone (NTBC, Orfadine®) as a therapeutic option. We have recently shown that selective screening is inadequate as initial symptoms are often uncharacteristic which leads to a considerable delay in diagnosis and treatment. This has a negative impact on morbidity and mortality as well as long-term outcome. For example, the odds ratio to develop hepatocellular carcinoma is 12.7 when treatment is initiated after the first birthday compared to start of treatment in the neonatal period...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28750872/a-new-index-predicts-early-allograft-dysfunction-following-living-donor-liver-transplantation-a-propensity-score-analysis
#16
Jiu-Lin Song, Jian Yang, Lu-Nan Yan, Jia-Yin Yang, Tian-Fu Wen, Bo Li, Yong Zeng, Hong Wu, Wen-Tao Wang, Ming-Qing Xu, Zhe-Yu Chen, Yong-Gang Wei, Li Jiang
OBJECTIVE/AIM: The aim of this study was to identify a new index to predict early allograft dysfunction following living donor liver transplantation. METHODS: The study enrolled 260 adult living donor liver transplantation recipients. Postoperative laboratory variables were assessed for their association with the prevalence of early allograft dysfunction using the inverse probability of treatment weighting and propensity-score matching (n=93 pairs) analysis. RESULTS: Forty-seven recipients (18...
June 23, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28737869/management-of-cirrhotic-ascites-in-children-review-and-recommendations-part-1-pathophysiology-diagnostic-evaluation-hospitalization-criteria-treatment-nutritional-management
#17
REVIEW
David F Bes, M Cristina Fernández, Ivone Malla, Horacio A Repetto, Daniel Buamsch, Susana López, Roxana Martinitto, Miriam Cuarterolo, Fernando Álvarez
Ascites is a major complication of cirrhosis. There are several evidence-based articles and guidelines for the management of adults, but few data have been published in relation to children. In the case of pediatric patients with cirrhotic ascites (PPCA), the following questions are raised: How are the clinical assessment and ancillary tests performed? When is ascites considered refractory? How is it treated? Should fresh plasma and platelets be infused before abdominal paracentesis to prevent bleeding? What are the hospitalization criteria? What are the indicated treatments? What complications can patients develop? When and how should hyponatremia be treated? What are the diagnostic criteria for spontaneous bacterial peritonitis? How is it treated? What is hepatorenal syndrome? How is it treated? When should albumin be infused? When should fluid intake be restricted? The recommendations made here are based on pathophysiology and suggest the preferred approach to its diagnostic and therapeutic aspects, and preventive care...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28728923/ruptured-sinus-of-valsalva-aneurysm-causing-acute-right-heart-and-hepatorenal-failure-requiring-urgent-surgery
#18
Benjamin Swinson, Robert Grant, Ekta Paw, Raibhan Yadav, Anand Iyer
A sinus of Valsalva aneurysm is a rare cardiac anomaly that may be congenital or acquired. Very rarely, they present with acute symptoms as a result of rupture into the cardiac chamber. If left untreated, they almost always result in deteriorating cardiac function. We report a patient presenting with acute right heart failure and hepatorenal syndrome requiring urgent surgery and double patch repair.
May 29, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28722461/-disorders-of-water-and-electrolyte-metabolism-and-changes-in-acid-base-balance-in-patients-with-ascitic-liver-cirrhosis
#19
Halima Gottfriedová, Miroslava Horáčková, Milena Čáslavská, Julius Špičák, Otto Schück
In patients with advanced cirrhosis with ascites disorders of water and electrolyte metabolism are often present and they are associated with changes in acid-base balance. These changes can be very complicated, their diagnosis and treatment difficult. Dilutional hyponatremia is the most common disorder. Hyponatremia in these patients is associated with increased morbidity and mortality before and after liver transplantation. Other common disorders include hyperchloremic acidosis, hypokalemia, metabolic alkalosis, lactic acidosis, respiratory alkalosis...
2017: Casopís Lékar̆ů C̆eských
https://www.readbyqxmd.com/read/28721347/peliosis-hepatis-complicating-pregnancy-a-rare-entity
#20
Muhammad Osama Butt, Nasir Hassan Luck, Syed Mujahid Hassan, Zaigham Abbas, Muhammed Mubarak
Peliosis hepatis (PH) is a rare, benign condition of the liver characterized by the presence of blood-filled lacunar spaces in the parenchyma. It usually has a chronic presentation and is a rare cause of portal hypertension reported in adult patients. Its etiology is diverse and ranges from infectious agents to tumors to toxic substances and anabolic steroids; however, the cause remains unclear in 25-50% of patients. Similarly, the symptomatology and imaging findings are diverse. Biopsy is the definitive test to diagnose the condition...
June 2017: Journal of Translational Internal Medicine
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